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What is dyspnea and what can be done about it? Dan Culver DO Outline What is dyspnea? What tests do we use for it? Questions What is dyspnea? What is dyspnea? Central Airways narrowing Acid build-up Difficulty in breathing or

  1. What is dyspnea and what can be done about it? Dan Culver DO

  2. Outline • What is dyspnea? • What tests do we use for it? • Questions

  3. What is dyspnea?

  4. What is dyspnea? Central Airways narrowing Acid build-up “Difficulty in breathing or Lung blood vessels in catching the breath” Low oxygen Muscle weakness delivery Restriction of lung motion

  6. The main role of the lungs is gas exchange

  7. Subpleura Interlobular septa Bronchovascular area

  8. Sarcoidosis likes to involve the airways too “Pebbly mural” appearance

  9. Bronchoscopy results LUL LLL Upper division RUL lingula

  10. Pulmonary hypertension in sarcoidosis 80 70 Percent with Pulmonary 60 Hypertension 50 40 30 20 10 0 Kyoto Detroit Milan New York Cincinnati Transplant All patients Only Dyspneic Patients

  11. Muscle weakness in sarcoidosis

  12. Muscle weakness relates to other variables affecting QOL 6MWD Hand grip Extensor Quadrice HPT Pi max ps PT Men -0.25 -0.25 -0.29 -0.17 -0.36 0.24 Women -0.12 -0.21 -0.30 -0.04 -0.043 0.051 Marcellis RG. ERJ 2011

  13. Outline • What is dyspnea? • What tests do we use for it? • Questions

  15. Spirometry Flow-volume loop showing obstruction

  16. Data at the time of evaluation

  18. The main role of the lungs is gas exchange

  21. Scadding X-ray stages Stage I: 85-90% Stage II: 50-65% Stage III: 20-30% Stage IV: 0%

  22. Which patient has worse pulmonary function tests? 69 year-old male 46 year-old male

  23. Some potential causes of shortness of breath in sarcoidosis • Involvement of the lung tissue • Airways involvement (usually asthma-like) • Deconditioning (out of shape) • Anemia (low blood counts) • Airways involvement with stenosis • Poor heart function • Muscle inflammation RARE • High blood pressures in the lungs (pulmonary hypertension)

  24. Chest x-ray is not a very good marker of disease activity 51% 25 23 20 n=36 ĸ=0.54 15 15 9 10 7 6 4 5 3 3 1 1 0 0 0 0 0 0 -7 -6 -5 -4 -3 -2 -1 0 1 2 3 4 5 6 7 Judson MA. Respirology 2008

  25. What about chest CT scan?

  26. Does ACE help? Correlation with respiratory functional impairment in 144 non-smoking patients Rothkrantz-Kos S. Clin Chem 2003

  27. Correlation of biomarkers with outcome Acute sarcoidosis Chronic sarcoidosis AUC 0.89 (0.79-1) sIL2R AUC 0.72 (0.52-0.92) for ACE Rothkrantz-Kos S. Clin Chem 2003

  28. Sarcoidosis penumbra Lazar CA. SRCCM 2010

  30. Pulmonary sarcoid with bulky adenopathy

  31. Overdosing steroids? • 36 acute exacerbations • Mean steroid dose 19 mg • FVC improved from 68% to 82% • Median assessment at 21 days Goldstein DS. Thorax 1986 McKinzie BP. AM J Med Sci 2010

  32. Predictors of requirement for therapy at 2 years in ACCESS 120 n=205 Independent predictors 100 Initial treatment No. of subjects 80 O.R. 3.6 (1.8-7.2) 60 Dyspnea O.R. 2-4 40 20 0 No early treatment Early treatment Baughman RP. QJM 2006

  34. Data at the time of evaluation

  35. Making the diagnosis: Bronchoscopy Overall diagnostic yield 70-97% Yield in non-chest diseases Sensitivity TBBx: 43-96% EBBx: 40-75% TBNA: 46-66% Combination: 91% “Pebbly mural” appearance

  36. Treatment • Treat the disease – Immunosuppressives – TNF antagonists variably successful • Cognitive behavioral therapy • Aerobic and strength training • Stimulants Hoving JL. Scand J Rheumatol 2009 Chauffier K. Rheumatology 2011 White PD Lancet 2011 Lower EE. Chest 2008

  38. Endobronchial stenosis requiring lung transplant

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